Localized cystic disease of the kidney

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Localized cystic disease of the kidney
Diagnosis in short

LM

cysts with cuboidal or flat lining epithelium - contiguous with renal collecting ducts;

renal parenchyma (renal tubules or glomeruli) with in septations; +/-foamy histiocytes; +/-refractile material within the cysts
LM DDx cystic renal tumours (cystic clear cell renal cell carcinoma, cystic nephroma, multilocular cystic renal neoplasm of low malignant potential), hereditary cystic renal disease (autosomal dominant polycystic kidney disease)
Site kidney

Signs +/-hematuria (uncommon), often incidentaloma
Symptoms abdominal pain - due to mass effect
Prevalence very rare
Radiology localized cystic disease, Bosniak classification 2 or 3
Prognosis benign
Clin. DDx cystic renal cell carcinoma
Treatment excision for diagnosis

Localized cystic disease of the kidney is a rare benign entity that should be separated from hereditary cystic kidney diseases and cystic renal tumours.[1]

It is also known as segmental cystic disease of the kidney, unilateral cystic disease of the kidney[1] and benign multilocular cyst of the kidney.[2]

General

  • Rare and benign.
  • No family history of polycystic kidney disease.

Presentation:

  • Usually an incidental finding (7 of 9 cases).
  • Hematuria - uncommon (2 of 9 cases).

Radiology:

Gross

Features:[1]

  • Focal cystic lesion(s) without a capsule involving the renal papillae +/-renal cortex.
    • Contain clear or light-yellow serous fluid.

Note:

  • Focal - only part of the kidney should be involved.

Microscopic

Features:[1]

  • Cysts with cuboidal or flat lining epithelium.
    • Contiguous with renal collecting ducts.
  • Renal parenchyma (renal tubules or glomeruli) with in (cyst) septations.
  • +/-Foamy histiocytes.
  • +/-Refractile material within the cysts.

Notes - negatives:

  • No significant inflammation.
  • No capsule.
  • No papillary hyperplasia.

DDx:

See also

References

  1. 1.0 1.1 1.2 1.3 Ding, Y.; Chen, L.; Deng, FM.; Melamed, J.; Fan, R.; Bonsib, S.; Zhou, M. (Apr 2013). "Localized cystic disease of the kidney: distinction from cystic neoplasms and hereditary polycystic diseases.". Am J Surg Pathol 37 (4): 506-13. doi:10.1097/PAS.0b013e318271eff9. PMID 23211292.
  2. 2.0 2.1 Bhat, S.; Thomas, A. (Oct 2007). "Benign multilocular cyst or multilocular cystic nephroma in adults: three case reports.". Indian J Surg 69 (5): 209-11. doi:10.1007/s12262-007-0025-1. PMID 23132986.